Özet
Anti-nötrofil sitoplazmik antikor (ANCA) asosiye vaskülitlerde (AAV), üst solunum yolu, deri, nörolojik sistem ve konstitüsyonel bulgularla birlikte kardiyak, renal ve pulmoner sistemleri tutan ve organ fonksiyon kaybı ya da yaşamı tehdit eden klinik tablolar gelişebilir. Bu nedenle erken tanı için hekimler arasında farkındalığın artırılması gerekmektedir. AAV şüphesi bulunan ve kutanöz vaskülit, kronik üst ve alt solunum yolu hastalıkları, hızlı ilerleyen böbrek fonksiyon bozukluğu, periferik nöropati gibi AAV tanısını düşündüren sistemik bulguları olan hastalarda, birincil tanı yöntemi olarak yüksek kaliteli antijen-spesifik yöntemle proteinaz-3 ANCA ve miyeloperoksidaz ANCA bakılmalıdır. AAV hastalarının vaskülitler konusunda deneyimli merkezler tarafından multidisipliner bir ekiple değerlendirilmesi akılcı yaklaşımdır. Teknolojik gelişmelerle birlikte tanı, ayırıcı tanı ve hastalık süresinin izleminde çeşitli laboratuvar, görüntüleme teknikleri ve girişimsel yöntemler kullanılmaktadır. Hastalık aktivitesinin ve organ hasarının değerlendirilmesinde çeşitli hastalık kompozit indekslerinden yararlanılmaktadır. AAV tedavisi, remisyon indüksiyonu ve idame tedavisi olmak üzere iki aşamada planlanmaktadır. Organ veya yaşamı tehdit eden olgularda, indüksiyon tedavisinde glukokortikoidlere ek olarak rituksimab veya siklofosfamid temelli rejimler önerilmektedir. Remisyon sağlandıktan sonra, nüksleri önlemek amacıyla idame tedavisine geçilir ve bu dönemde en sık tercih edilen ajan rituksimabdır. Glukokortikoidler, indüksiyon tedavisinin temel bileşenlerinden biri olmasına karşın, yapılan çalışmalar düşük doz glukokortikoid rejimlerinin standart dozlara benzer etkinlik gösterdiğini ve daha düşük enfeksiyon riski ile ilişkili olduğunu ortaya koymuştur. Ayrıca, rituksimab ve mepolizumab gibi biyolojik ilaçların kullanımıyla birlikte glukokortikoidler ve diğer immünsüpresiflere bağlı gelişen hasar gelişimi önemli oranda azalmıştır. Hastalar takipleri sırasında tedavi ilişkili yan etkiler ve komorbiditeler (hipertansiyon, osteoporoz, kardiyovasküler hastalıklar) açısından periyodik olarak taranmalı ve hastalara gerekli yaşam tarzı değişiklikleri önerilmelidir.
Anahtar Kelimeler:
Vaskülit, granülomatöz polianjiitis (GPA), eozinofilik granülomatöz polianjiitis (EGPA), mikroskopik polianjiitis (MPA), immünosüpresif tedavi, siklofosfamid, rituksimab, plazma değişimi, glukokortikoid, komorbidite, enfeksiyonlar
Kaynaklar
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